To the Editor: A 63-year-old woman presented with polyuria, polydipsia, lethargy and vomiting. Two weeks previously, she had been diagnosed as having diffuse scleroderma with possible interstitial lung disease, and had started taking 50 mg prednisolone daily. Her past history included diabetes, hypertension, hypercholesterolaemia and β-thalassemia trait, and her other medications were metformin, glibenclamide, quinapril and amlodipine.

Examination revealed blood pressure 150/60 mmHg, a loud second heart sound with no murmurs, and late inspiratory crepitations at lung bases. Her serum creatinine concentration was 270 μmol/L (compared with 100 μmol/L two weeks previously) and serum glucose concentration was 26.5 mmol/L. Treatment by the admitting doctor included insulin, rehydration, and cessation of prednisolone (given hyperglycaemia) and quinapril (secondary to acute renal impairment). She developed a fever and cough, with bilateral pneumonia, which was treated with intravenous ceftriaxone.

Despite normotension, concern regarding scleroderma renal crisis (SRC) was raised. On Day 12 of admission, when renal failure had developed to the dialysis-dependent level (serum creatinine level, 690 μmol/L), quinapril was recommenced for its proposed renoprotective effect and haemodialysis was initiated. Microangiopathic haemolytic anaemia (haemoglobin, 7.2 g/L) was diagnosed, with fragmented red blood cells (Box).

Several months later, she continues on haemodialysis three times a week. Renal biopsy was not performed given the clinical picture of diffuse scleroderma and recent corticosteroid use with rapid development of renal failure — consistent with SRC.

SRC is defined as rapidly progressive renal failure and/or new onset of malignant hypertension during the course of scleroderma, occurring in 15%–20% of patients with the diffuse variety.1 Risk factors include male sex, black race, and early diffuse scleroderma with rapidly progressive skin thickening.2 Precipitation of SRC by corticosteroid use, especially in normotensive patients, is well described, particularly with high-dose (>15 mg/day) treatment.2

Early diagnosis is critical because treatment may preserve renal function.3 Outcomes have improved with use of angiotensin-converting enzyme (ACE) inhibitors,2 which are thought to improve renal function by controlling the high renin levels seen in patients with SRC. About 61% of patients have a good outcome, with no or temporary dialysis.3 Predictors of poor outcome, despite ACE inhibitor use, include older age, male sex, higher initial serum creatinine level, and scleroderma myocardial disease.1 Eleven per cent of SRC patients remain normotensive and have significantly reduced 12-month survival rates.4 This may relate to delay in diagnosis of SRC.

The use of high dose corticosteroids in patients with early diffuse scleroderma should be strongly discouraged, and intensive monitoring for SRC is recommended if low dose corticosteroids are required.

Peripheral blood film, magnification x40

Changes of thalassaemia (microcytosis and hypochromasia) and microangiopathic haemolysis (fragmented red cells and spherocytes). 1. Spherocytes. 2. Fragmented red blood cells.